HEME BIOSYNTHETIC PATHWAY (PW:0000218)

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Description

Heme serves as a prosthetic group for enzymes and proteins involved in a broad range of important cellular processes such as oxygen transport and storage, photosynthesis, redox, signaling and drug metabolism. Iron-protoporphyrin IX, or heme b, is the parent molecule whose function depends on the associated polypeptide, and its modifications yield other forms of heme. Four pyrroles linked by a methine bridge form the planar tetrapyrrole unit of heme; each pyrrole coordinates the iron atom via a n

Pathway Diagram:

Ariadne Genomics Inc. Urod uroporphyrinogen III ---> coproporphyrinogen III Alad 5-aminolevulinate ---> porphobilinogen Fech ---> H+ H+ Hmbs ---> NH3 NH3 Urod ---> , Uros ---> . . Alas2 ---> CO2 protoporphyrin IX Fe2+ ---> Fech Uros succinyl- coenzyme A Hmbs L-glycine 5-aminolevulinate porphobilinogen hydroxymethylbilane uroporphyrinogen III coproporphyrinogen III Fe2+ heme protoporphyrinogen IX CO2 Fech Ppox citrate cycle pathway ---> succinyl- coenzyme A citrate cycle pathway protoporphyrin IX ---> heme protoporphyrinogen IX ---> protoporphyrin IX Cpox coproporphyrinogen III ---> protoporphyrinogen IX , Alas2 Alas1 hydroxymethylbilane ---> uroporphyrinogen III porphobilinogen ---> hydroxymethylbilane L-glycine ---> 5-aminolevulinate
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Genes in Pathway:

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heme biosynthetic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Alad aminolevulinate dehydratase JBrowse link 5 78,368,867 78,379,206 RGD:1578396
RGD:11554188
RGD:4144542
G Alas1 5'-aminolevulinate synthase 1 JBrowse link 8 114,927,704 114,941,038 RGD:1578396
RGD:11554188
RGD:4144542
RGD:1601233
G Alas2 5'-aminolevulinate synthase 2 JBrowse link X 23,167,576 23,187,356 RGD:1578396
RGD:11554188
RGD:4144542
G Cpox coproporphyrinogen oxidase JBrowse link 11 44,039,665 44,049,648 RGD:1578396
RGD:11554188
RGD:4144542
G Fech ferrochelatase JBrowse link 18 59,941,992 59,975,192 RGD:1578396
RGD:11554188
RGD:4144542
G Hmbs hydroxymethylbilane synthase JBrowse link 8 48,667,278 48,674,673 RGD:1578396
RGD:11554188
RGD:4144542
RGD:2301704
G Ppox protoporphyrinogen oxidase JBrowse link 13 89,650,094 89,654,998 RGD:1578396
RGD:11554188
RGD:4144542
RGD:1599180
G Slc25a38 solute carrier family 25, member 38 JBrowse link 8 128,790,348 128,802,988 RGD:11554188
RGD:11556278
G Tspo translocator protein JBrowse link 7 124,460,358 124,470,610 RGD:11554188
G Urod uroporphyrinogen decarboxylase JBrowse link 5 135,855,429 135,859,515 RGD:1578396
RGD:11554188
RGD:4144542
RGD:2303399
G Uros uroporphyrinogen III synthase JBrowse link 1 205,755,766 205,778,170 RGD:1578396
RGD:4144542
RGD:4144823
RGD:11554188

Pathway Gene Annotations

Disease Annotations Associated with Genes in the heme biosynthetic pathway
Disease TermsGene Symbols
Abdominal PainHmbs
Acute Kidney InjuryAlad
AnemiaAlad , Alas2
Anemia, HemolyticAlas2
Anemia, HypochromicAlas2 , Slc25a38
Anemia, Sickle CellAlad , Hmbs
Anemia, SideroblasticAlad , Alas2 , Slc25a38
Anemia, Sideroblastic Spinocerebellar AtaxiaAlas2
Anemia, Sideroblastic, Pyridoxine-Refractory, Autosomal RecessiveSlc25a38
Arsenic PoisoningAlad
Arthritis, ExperimentalCpox
Bile Duct DiseasesFech
BlisterPpox
Breast NeoplasmsUrod
Carcinoma, Renal CellAlad
Colorectal NeoplasmsPpox
Congenital Disorders of GlycosylationHmbs
CoproporphyriaCpox
Coproporphyria, HereditaryCpox
Diabetes Insipidus, NeurogenicPpox
Diabetes Mellitus, ExperimentalAlad , Alas1 , Hmbs
End Stage Liver DiseaseTspo
FatigueAlad
FibrosisFech
Focal Dermal HypoplasiaHmbs
Genetic Diseases, InbornHmbs
Genetic Predisposition to DiseaseAlad
HarderoporphyriaCpox
Head and Neck NeoplasmsUrod
Hematologic DiseasesAlad
HemochromatosisAlad , Alas2
Hepatic EncephalopathyTspo
HyperbilirubinemiaAlas2 , Hmbs
HypercholesterolemiaHmbs
HypertensionAlad
Hypertension, PulmonaryAlas1
HypothyroidismAlad
JaundiceFech
Kidney DiseasesAlad
Lead PoisoningAlad
Lead Poisoning, Nervous SystemAlad
Liver CirrhosisAlad
Liver Cirrhosis, ExperimentalTspo
Liver DiseasesAlad , Cpox
Liver Neoplasms, ExperimentalAlas2
Manganese PoisoningAlad
MeningiomaAlad
Porphyria Cutanea TardaAlad , Cpox , Urod
Porphyria, Acute HepaticAlad
Porphyria, Acute IntermittentCpox , Hmbs , Ppox
Porphyria, Acute Intermittent, Nonerythroid VariantHmbs
Porphyria, ErythropoieticAlas2 , Fech , Uros
Porphyria, HepatoerythropoieticUrod
Porphyria, South African TypePpox
Porphyria, VariegatePpox
PorphyriasAlas1
Porphyrias, HepaticAlad , Cpox , Fech , Hmbs , Ppox , Urod
Prostatic NeoplasmsAlad , Hmbs
Protoporphyria, ErythropoieticFech
Protoporphyria, Erythropoietic, X-Linked DominantAlas2
Respiratory Distress Syndrome, AdultAlad
RhabdomyolysisAlas1
SeizuresAlad
SeminomaHmbs
SepsisAlas1
Status EpilepticusAlad
Urinary Bladder NeoplasmsHmbs
VomitingHmbs
X-Linked Sideroblastic AnemiaAlas2 , Slc25a38
Pathway Annotations Associated with Genes in the heme biosynthetic pathway

References Associated with the heme biosynthetic pathway:

Ontology Path Diagram:

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